Kawasaki Disease or Incomplete Kawasaki Disease Clinical Pathway — Emergency Department and Inpatient

Abnormal Echocardiogram Subsequent Treatment

Guidelines for assessment and management for patients of all ages with abnormal echocardiogram results (any coronary artery Z score of ≥ 2.5)

For all patients, monitor and manage as indicated based on response to initial treatment.

All Patients with Abnormal Echo	Start prednisolone 2 mg/kg divided BID x 5, days then taper (if not already started on steroids) Consult Rheumatology
Response to Initial Treatment	Management and/or Treatment
Resistant Fever > 38.0 OR lingering clinical symptoms > 24-36 hours after completion of IVIG	Re-check labs (CBC, CRP, LFTs) before escalating therapy Consult Rheumatology to guide infliximab therapy Infliximab 10 mg/kg Repeat labs (as above) 24 hours after completing infliximab treatment Discuss timing of repeat echo with Cardiology
	If persistent fevers/symptoms after treatment with infliximab: Re-treat with IVIG Consider pulse methylprednisolone 30 mg/kg/dose (max 1 g) every 24 hrs x 3 days Monitor CBC, CRP, LFTs, reticulocyte count, LDH after 24 hours
Responsive Afebrile < 38.0 AND clinically improving ~ 48 hours after completion of IVIG (or adjunctive treatment)	Consider discharge, but discuss with Cardiology and Rheumatology. Consider repeat echocardiogram prior to discharge if significant coronary findings.

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The clinical pathways are based upon publicly available medical evidence and/or a consensus of medical practitioners at The Children’s Hospital of Philadelphia (“CHOP”) and are current at the time of publication. These clinical pathways are intended to be a guide for practitioners and may need to be adapted for each specific patient based on the practitioner’s professional judgment, consideration of any unique circumstances, the needs of each patient and their family, and/or the availability of various resources at the health care institution where the patient is located.

Accordingly, these clinical pathways are not intended to constitute medical advice or treatment, or to create a doctor-patient relationship between/among The Children’s Hospital of Philadelphia (“CHOP”), its physicians and the individual patients in question. CHOP does not represent or warrant that the clinical pathways are in every respect accurate or complete, or that one or more of them apply to a particular patient or medical condition. CHOP is not responsible for any errors or omissions in the clinical pathways, or for any outcomes a patient might experience where a clinician consulted one or more such pathways in connection with providing care for that patient.